Biomedical subjects
Publications and source records attributed to N S Proctor.
Gliomas of the optic nerve are rare, comprising only 1% of intracranial neoplasms in adults and 5% in children. This is the first recorded case of a ganglioglioma of the optic nerve. A 2-year-old White boy presented with a convergent squint and blindness of the left eye. At operation a pale, firm, fusiform swelling was found. The cytological and histopathological features are described.
The incidence of primary ocular malignant melanoma in blacks during the last 25 years in a large part of the Transvaal Province of South Africa is reported. Only 8 cases were diagnosed, 1 uveal, 4 conjunctival, and 3 orbital. During this same period 153 cases of primary ocular malignant melanoma were diagnosed in whites. The rarity of these tumours among blacks is stressed and the reasons for this infrequent incidence discussed.
We report 2 cases of demyelinating disease of unusually late onset in a mother and her son. The late onset of symptoms, at the age of 63 years in the mother's case, is rare. The onset in the son was at the age of 35 years. The relatively few adult cases previously reported range in age from 25 to 65 years, but only 2 cases in persons over the age of 60 could be found in the literature. The clinical and neuropathological findings are described.
A case of centronuclear myopathy of adult onset with striking hypertrophy of lower limb muscles in a black South African man is described with details of the light microscopic, histochemical, and ultrastructural features. The association of hypertrophied muscles with centronuclear myopathy has not to our knowledge been reported previously and it is felt that this may be a variant of the condition.
Centronuclear myopathy occurring sporadically in two African female children is reported, with details of clinical history and histological, histochemical, and ultrastructural findings, and a review of 58 previously reported cases. In spite of distinctive histological features, the clinical presentation of this condition is variable, there are different modes of inheritance, and the pathogenesis remains unclear.
We report a case of Alexander's disease in a black South African boy of 9 years. The child presented with a grossly abnormal stooped posture, generalised weakness, and slurred nasal speech. Computerised tomography revealed diffuse low radio-density confined to the white matter, and enlarged ventricles. Cerebral biopsy confirmed the diagnosis. The histopathological and electron microscopic features are essentially the same as those described in previous reports. We note the presence of dense clumps of material within some of the Rosenthal fibres, and can find no reference in the literature describing this. Sural nerve biopsy was normal.
Haemangiopericytoma is a very vascular tumour which is slow growing and often painless. It is important to realise the malignant potential of these tumours and to treat them by wide surgical excision. Angiography was helpful in this case because it gave warning of probable malignancy.
The clinical and histopathological features of a case of solitary extramedullary plasmacytoma of the breast are described. After 46 mth of follow-up, there has been no recurrence of the tumour, and no evidence of further extramedullary plasmacytomas, multiple myeloma or diffuse myelomatosis either clinically, radiologically or on biochemical and haematological investigations. She has developed diabetes mellitus. Distinction is made between the true extramedullary plasmacytoma and those which are a manifestation of multiple myeloma. The unpredictable behaviour and prognosis of extramedullary plasmacytomas is indicated.
An intracerebral Schwannoma giving rise to temporal lobe epilepsy is described. The possible origin of such tumours in the central nervous system in general, and in the temporal lobe in particular, is discussed.
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